Please use this identifier to cite or link to this item: http://hdl.handle.net/10261/32922
Share/Export:
logo share SHARE logo core CORE BASE
Visualizar otros formatos: MARC | Dublin Core | RDF | ORE | MODS | METS | DIDL | DATACITE
Title

Human TRAF3 adaptor molecule deficiency leads to impaired Toll-like receptor 3 response and susceptibility to herpes simplex encephalitis

AuthorsPérez de Diego, Rebeca; Sancho-Shimizu, Vanessa; Lorenzo, Lazaro; Puel, Anne; Plancoulaine, Sabine; Picard, Capucine; Herman, Melina; Cardon, Annabelle; Durandy, Anne; Bustamante, Jacinta; Vallabhapurapu, Sivakumar; Bravo, Jerónimo CSIC ORCID ; Warnatz, Klaus; Chaix, Yves; Cascarrigny, Françoise; Lebon, Pierre; Rozenberg, Flore; Karin, Michael; Tardieu, Marc; Al-Muhsen, Saleh; Jouanguy, Emmanuelle; Zhang, Shen-Ying; Abel, Laurent; Casanova, Jean-Laurent
KeywordsHerpes simplex encephalitis (HSE)
TRAF3
Herpes simplex virus 1 (HSV-1)
TLR3
Interferon (IFN)
Issue Date9-Sep-2010
PublisherElsevier
CitationImmunity 33(3):400-11 (2010)
AbstractTumor necrosis factor (TNF) receptor-associated factor 3 (TRAF3) functions downstream of multiple TNF receptors and receptors that induce interferon-alpha (IFN-alpha), IFN-beta, and IFN-lambda production, including Toll-like receptor 3 (TLR3), which is deficient in some patients with herpes simplex virus-1 encephalitis (HSE). Mice lacking TRAF3 die in the neonatal period, preventing direct investigation of the role of TRAF3 in immune responses and host defenses in vivo. Here, we report autosomal dominant, human TRAF3 deficiency in a young adult with a history of HSE in childhood. The TRAF3 mutant allele is loss-of-expression, loss-of-function, dominant-negative and associated with impaired, but not abolished, TRAF3-dependent responses upon stimulation of both TNF receptors and receptors that induce IFN production. TRAF3 deficiency is associated with a clinical phenotype limited to HSE resulting from the impairment of TLR3-dependent induction of IFN. Thus, TLR3-mediated immunity against primary infection by HSV-1 in the central nervous system is critically dependent on TRAF3
Description11 pages, 6 figures.- PMID: 20832341 [PubMed]PMCID: PMC2946444 [Available on 2011/9/1]
Publisher version (URL)http://dx.doi.org/10.1016/j.immuni.2010.08.014
URIhttp://hdl.handle.net/10261/32922
DOI10.1016/j.immuni.2010.08.014
ISSN1074-7613
E-ISSN1097-4180
Appears in Collections:(IBV) Artículos




Files in This Item:
File Description SizeFormat
Immunity 033-00400.pdf1,01 MBAdobe PDFThumbnail
View/Open
Show full item record
Review this work

PubMed Central
Citations

143
checked on May 25, 2022

SCOPUSTM   
Citations

249
checked on May 20, 2022

WEB OF SCIENCETM
Citations

233
checked on May 23, 2022

Page view(s)

595
checked on May 26, 2022

Download(s)

359
checked on May 26, 2022

Google ScholarTM

Check

Altmetric

Dimensions


Related articles:


WARNING: Items in Digital.CSIC are protected by copyright, with all rights reserved, unless otherwise indicated.